Primary Plasma Therapy For Thrombotic Thrombocytopenic Purpura

A 25-year old male was admitted for an episode of right sided headache and subsequent generalized seizure. On admission his temperature was 37.6°. He had generalized petechiae and conjunctival hemorrhages. Organomegaly and lymphadenopathy were absent. There was mild left sided weakness. The Hgb. was 6.9 g/dl., reticulocyte count 10%, WBC 11,500/mm3, and platelet count 10,000/mm3. There were numerous schistocytes on the peripheral smear; bone marrow revealed panhyperplasia. Coagulation studies were normal. The BUN was 30, and the creatinine 1.7 mg/dl. Plasma was positive for Hgb. CT scan was negative for gross intracranial bleeding. The diagnosis of T.T.P. was made. On admission, the patient received 10 units of platelets and 2 units of packed red blood cells. He did not require further red cell or platelet transfusions during the rest of his hospital course. He was then started on infusions of fresh-frozen plasma. He then received one unit every 3 hours for 6 days, one unit every 6 hours for 2 days, then one unit every 12 hours for 2 days and finally 1 unit daily for 5 days. The response was immediate. After the infusions were started, the hematologic parameters steadily improved. The patient’s hematuria rapidly improved. Further CNS symptoms did not appear. The patient’s Hgb. was 12 g/dl, and reticulocyte count was 2.5% by the 9th day. His platelet count was normal by the 4th day. The patient was discharged on the 15th day. Infusions of plasma were discontinued at the time of discharge. The patient required plasma therapy 4 weeks later for recurrent thrombocytopenia (50,000/mm3). The patient has remained normal for 9 months since therapy and further plasma has not been required. Primary plasma therapy for T.T.P. as sole treatment should be further studied.